Ebstein anomaly (EA) is a rare congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, with atrialization of the right ventricle and variable degree of malformation and displacement of the anterior leaflet. Patients can have a variety of symptoms related to the anatomic abnormalities of EA and their hemodynamic effects or associated structural and conduction system disease. Most frequently cyanosis, palpitations, fatigue and dyspnea can be observed. In severe forms edema and ascites. Treatment of EA is complex and depends of the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Options of treatment include medical therapy, radiofrequency ablation, and surgical therapy. Main aim of the surgical therapy is to correct the underlying tricuspid valve, right ventricular abnormalities and any associated intracardiac defects if exist. Palliative procedures and cardiac transplantation can be considered in most sever EA patients. JRCD 2013; 1 (4): 139–143